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Acquired long QT syndrome in a child undergoing craniotomy: a case report
Y C Hsieh1, L H Chow, T D Wong
1Department of Anesthesiology, Veterans General Hospital-Taipei, Taiwan, R.O.C.
Insights
Acquired long QT syndrome (LQTS) can be fatal in children, even with prompt treatment. Differentiating between congenital and acquired LQTS is crucial for effective management and improved outcomes in pediatric cardiac care.
Area of Science:
- Pediatric Cardiology
- Clinical Electrophysiology
- Anesthesiology
Background:
- Long QT syndrome (LQTS) is a rare cardiac conduction disorder in children, potentially causing severe arrhythmias, syncope, and sudden death.
- Acquired LQTS is often linked to electrolyte imbalances and specific medications like procainamide.
Observation:
- A case of acquired LQTS in a pediatric patient undergoing craniotomy is presented.
- Ventricular tachycardia (torsade de pointes) with a prolonged QT interval occurred post-anesthesia induction.
- Arrhythmia worsened with propranolol but was controlled with isoproterenol, suggesting acquired LQTS.
Findings:
- Prompt diagnosis and differentiation between congenital and acquired LQTS are critical for patient outcomes.
- While isoproterenol successfully managed the arrhythmia, the prolonged anesthesia duration contributed to the fatal outcome.
- This case highlights the challenges in managing LQTS during complex surgical procedures.
Implications:
- Emphasizes the importance of vigilant cardiac monitoring and rapid differential diagnosis in pediatric patients with suspected LQTS.
- Underscores the need for tailored anesthetic management and treatment strategies for LQTS patients.
- Suggests that timely and accurate diagnosis of LQTS type is paramount for effective therapeutic interventions and survival.
Abstract:
The long QT syndrome (LQTS) is a rare disorder of cardiac conduction in children. It may lead to severe arrhythmia, syncope or sudden death. Electrolyte disturbance and the use of procainamide are closely related to the acquired LQTS. This report concerns a fatality of acquired LQTS in a boy undergoing craniotomy. After induction of anesthesia, his electrocardiogram displayed a frequent torsade de pointes type of ventricular tachycardia with prolonged QT interval. Therefore, congenital LQTS was impressed. However, the arrhythmia was aggravated by intravenous propranolol. Isoproterenol infusion was given under the diagnosis of acquired LQTS. The arrhythmia was successfully abolished and a stable hemodynamic status was maintained. However, the child could not tolerate the anesthesia which lasted for 30 hours, and eventually succumbed, despite aggressive treatment and resuscitation attempts. Differential diagnosis between the types of LQTS should be made promptly. This may be of paramount importance in the outcome since prognosis depends upon proper treatment. This case report discusses the etiology and management of this rare abnormality.